Fatty acid oxidation disorders (FODs) can cause serious clinical manifestations or even death. There have been a variety of inherited metabolic FODs identified which are enzyme deficiencies, and there are also various acquired metabolic derangements manifested by inadequate energy reaching particular muscles, such as the heart under stress conditions. In addition, normal individuals experience nutritional inefficiencies due to metabolism of the food choices that they make.
Previously, the use of a seven carbon fatty acid was found to be effective in a method for treating an inherited disorder in at least one enzyme involved in fatty acid metabolism. This use was described and claimed in U.S. Ser. No. 09/890,559, filed Aug. 1, 2001 which is hereby incorporated by reference. U.S. Ser. No. 09/890,559 claims priority to U.S. Provisional Application 60/119,038 filed 5 Feb. 1999 and PCT/US00/03022 filed 3 Feb. 2000. In that work, seven carbon fatty acids were also found to be effective as an energy source for humans not suffering from an enzyme deficiency, but in need of nutrients that provide fuel for a metabolic pathway that is underutilized due to the lack of odd chain fatty acids in normal foodstuffs.
For individuals suffering from Medium Chain Acyl-CoA Dehydrogenase (MCAD) Deficiency, an inherited metabolic disorder characterized by a deficiency of the enzyme medium chain acyl-CoA dehydrogenase, there remained a need for a treatment other than the administration of seven carbon fatty acids. MCAD participates in the initial oxidation of seven carbon fatty acids; therefore deficiency of MCAD cannot be treated with seven carbon fatty acids. Conventional dietary therapy for MCAD patients is to eat a high carbohydrate, fat restricted diet, avoiding fasting and eating often throughout the day. However, there is no reliable parenteral approach for rescue of MCAD patients during crisis. Further, dietary control is more difficult with infants since some of the enzymes needed by humans to metabolize certain carbohydrates, such as starches, do not become active until nearly six months of age. Cornstarches may be useful for treatment of babies and children above six months of age, but may cause undesirable effects such as constipation.
It has now been found that five carbon fatty acids are useful as a treatment for MCAD. Further, five carbon fatty acids can be used to treat long-chain FODs that are also treatable with seven carbon fatty acids. Five carbon fatty acids present a metabolic profile differing from that presented upon administration of seven carbon fatty acids, and this may be advantageous in some circumstances.
Further, it has been found that a fifteen carbon fatty acid (C15) can be administered as a precursor to five carbon fatty acids in normal humans and animals and for certain metabolic disease states such as Short-chain acyl-CoA dehydrogenase (SCAD) deficiency, and are preferred to C5 fatty acids for oral administration. C5 fatty acids have been found to have an undesirable taste due to breakdown by enzymes present in the saliva, and such enzymes are not active on C15 compounds and therefore C15 has a more pleasant taste.